The amount of bile duct loss at the time of diagnosis appears to closely correlate with prognosis in patients with primary biliary cholangitis (PBC), according to a recently published study in Hepatology Research.
While there have been therapeutic advances in the last decades, a subset of patients still progress to liver‐related complications despite apparently mild disease at diagnosis.
PBC reflects an immune-mediated attack on small intrahepatic bile ducts, a network of tubes located inside the liver that collects and transports bile from the liver. The destruction of these ducts leads to bile accumulation in the liver (cholestasis); chronic cholestasis can in turn lead to cirrhosis and serious liver-related events (LREs).
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To further identify predictive factors of severity in patients with the disease, investigators retrospectively analyzed 274 biopsy-proven, treatment-naïve PBC patients managed at two Japanese centers between 1978 and 2021. All underwent standardized follow-up every 3-6 months.
The microscopic cellular appearance of the tissues (histology) was reviewed by an experienced hepatopathologist using the Nakanuma staging/grading system. Clinical severity was assessed through the albumin–bilirubin (ALBI) grade.
The results showed that 19 patients developed LREs, such as hepatocellular carcinoma and refractory ascites. Among those classified as “clinical early-stage” (ALBI grade 1), statistical analysis identified that having a Nakanuma bile duct loss score ≥1 and higher total bilirubin were both independent predictors of LREs.
Cumulative LRE incidence at 10, 20 and 30 years was 3.0%, 7.4% and 13.6%, respectively, with significantly higher event rates in patients showing any bile duct loss versus none. AUROC analysis suggested bile duct loss outperformed ALBI, FIB-4 and bilirubin for predicting LREs in this subgroup.
Patients with bile duct loss at early stages had higher levels of enzymes related to liver damage at baseline, and had more LREs and worse overall survival. Notably, the prognostic separation by bile duct loss also held among UDCA non-responders accessible by Paris II criteria.
“Our study demonstrated the clinical potential of evaluating bile duct loss score for estimating prognosis in patients with early‐stage PBC,” the authors concluded. “Our results suggest that if clinicians perform liver biopsy on PBC patients, especially at the early stage, considering bile duct loss score may help determine prognosis.”
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